RESUMEN
Objective: This review was conducted to define the natural history of unoperated Beckwith-Wiedemann syndrome (BWS) macroglossia and the effect of tongue reduction surgery upon breathing, eating, speaking and dentoskeletal development in individuals having BWS. Design: This is a retrospective study of medical records. SETTING: All patients were evaluated and treated in one of two Children's Hospitals by an ACPA approved Craniofacial Team. PATIENTS/PARTICIPANTS: Medical records were reviewed of 526 individuals having a diagnosis of BWS and evaluated in-person by a single craniofacial surgeon between 1986 and 2014 in conjunction with a series of multi-disciplinary craniofacial team colleagues. 28 individuals were excluded having had multiple tongue reductions elsewhere. 498 individuals comprise the "pre tongue-reduction group". The "post tongue-reduction group" consists of 391 individuals who underwent surgical tongue reduction by one surgeon using one technique between 1986 and 2014. MAIN OUTCOME MEASURES: The primary outcome measure was change in anterior dental occlusion following tongue reduction surgery. Tongue reduction surgery was performed on the assumption that it would improve dentoskeletal relationships. Secondary outcome measures were: breathing, feeding/swallowing, and speech. Results: A significant difference (p<0.001) over time between the two groups was found with less anterior occlusal abnormality in the tongue reduction group. Tongue reduction surgery had no mortality and minimal morbidity for breathing, feeding/swallowing, and speech and can ameliorate obstructive sleep apnea. Conclusions: Surgical tongue reduction for BWS macroglossia is recommended for the infant or child in primary dentition with a grossly abnormal anterior tooth/jaw relationship and/or obstructive sleep apnea.
Asunto(s)
Síndrome de Beckwith-Wiedemann , Macroglosia , Macroglosia/congénito , Apnea Obstructiva del Sueño , Niño , Lactante , Humanos , Macroglosia/cirugía , Estudios Retrospectivos , Lengua/cirugía , Síndrome de Beckwith-Wiedemann/complicaciones , Síndrome de Beckwith-Wiedemann/cirugía , Apnea Obstructiva del Sueño/cirugíaRESUMEN
PURPOSE: Idiopathic macroglossia is a rare entity of true tongue enlargement without an underlying etiology. There are only a few case reports on the diagnosis and management of idiopathic macroglossia. This study's purpose was to present a series of patients with idiopathic macroglossia and suggest a treatment algorithm. METHODS: This was a retrospective case series of a cohort of patients with macroglossia who were treated by the Oral and Maxillofacial Surgery service at the University of Texas Health Science Center at Houston (UTHealth)and Emory University. The patient's medical comorbidities, history of present illness, clinical presentation, radiographic findings, and disease management were studied. The outcome variables include normalization of the tongue size, dependence on parenteral nutrition, and tolerating tracheostomy decannulation. RESULTS: Five patients with a mean age of 45 years were included in the study. All of the patients (n = 5, 100%) in our cohort developed macroglossia following prolonged oral intubation, with 3.5 weeks being the average length of intubation. All patients presented with difficulty feeding orally and breathing. The average tongue dimension was 12.20 x 6.25 cm. All tongue enlargements were located in the anterior 2/3 of the tongue, and all patients had displaced anterior dentition. In addition, 60% of the patients (n = 3) experienced altered tongue sensation (pain and/or decreased taste). These patients were surgically managed with tracheostomy and percutaneous endoscopic gastrostomy (PEG) tube placement followed by partial glossectomy (n = 5, 100%). We defined successful outcomes as 1) modifying the tongue to a functional, nonprotruding form, 2) tracheostomy decannulation and 3) PEG tube removal. Tracheostomy decannulation and PEG tube removal were achieved in 80% of the patients (n = 4). CONCLUSIONS: In this patient cohort, we were unable to identify the cause of the pathology based on existing clinical data. When the etiology is unclear or irreversible, management should involve tracheostomy and surgical feeding access for the initial stabilization, followed by modified glossectomy to improve form, function, and cosmesis thereby improving the overall quality of life.
Asunto(s)
Macroglosia , Humanos , Persona de Mediana Edad , Macroglosia/etiología , Macroglosia/cirugía , Estudios Retrospectivos , Calidad de Vida , Glosectomía/métodos , AlgoritmosRESUMEN
OBJECTIVES: The role of tongue reduction surgery (TRS) in preventing excessive mandibular growth and anterior open bite in children with Beckwith-Wiedemann Spectrum (BWSp) is still controversial. This cross-sectional study aimed at comparing craniofacial growth pattern in children affected by BWSp either treated or not treated with early TRS for severe macroglossia. Considering the invasive nature of such surgery, the present study could help in clarifying the need for TRS to reduce or prevent growth disturbances. MATERIALS AND METHODS: Orthopantomography and lateral skull x-ray images were taken either from surgically treated or non-surgically treated patients, aged 5 to 8 years, to compare dentoskeletal features and craniofacial growth by cephalometric analysis. Molecular testing results were collected from their medical records. RESULTS: Eighteen BWSp patients were consecutively recruited: 8 underwent TRS at 14.9 ± 2.2 months of age, while 10 did not. Anterior open bite and dental class III were more frequently observed in the surgically treated group, but none showed skeletal class III. No statistically significant differences were observed in growth pattern, but children treated with TRS showed a tendency towards both maxillary and mandibular prognathism with protruding lower lip. Growth pattern seemed to be not related to molecular subtypes. CONCLUSIONS: These preliminary data suggest that early TSR does not improve craniofacial growth pattern and dentoskeletal features in BWSp children. CLINICAL RELEVANCE: Reductive glossectomy may not be justified for preventing or avoiding oro-facial deformities in BWSp; therefore, early monitoring of maxillofacial development of each affected child has a great clinical significance.
Asunto(s)
Síndrome de Beckwith-Wiedemann , Macroglosia , Mordida Abierta , Niño , Humanos , Síndrome de Beckwith-Wiedemann/cirugía , Mordida Abierta/cirugía , Estudios Transversales , Lengua/cirugía , Macroglosia/cirugíaRESUMEN
While traditionally in the realm of otorhinolaryngology or oral maxillofacial surgery, conditions involving the tongue may also be managed by plastic surgeons. The authors present an unusual case of acquired macroglossia resulting from angiotensin-converting enzyme inhibitor-induced angioedema and review the literature to discuss its surgical management from a plastic surgery perspective. A 62-year-old female suffered severe airway obstruction, respiratory arrest, and anoxic brain injury from angioedema-associated macroglossia. After tracheostomy, the patient was managed nonsurgically, with bite wound care and medications to minimize angioedema to marginal effect. Ultimately, a partial glossectomy was planned. The edematous distal tongue was amputated and closed primarily. On postoperative day 2, she was successfully weaned off mechanical ventilation and no longer suffers trauma from tongue biting. The simple anterior tongue resection described in this paper was an appropriate approach for our patient. More research is needed to guide plastic surgeons in an optimal approach for clinical scenarios.
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Angioedema , Macroglosia , Femenino , Humanos , Persona de Mediana Edad , Macroglosia/etiología , Macroglosia/cirugía , Lengua/cirugía , Glosectomía/métodos , Angioedema/cirugíaRESUMEN
While macroglossia is a newly accepted component of brachycephalic obstructive airway syndrome (BOAS) in dogs, macroglossia with increased tongue fat is a well-known cause for obstructive sleep apnea (OSA) in people, and targeted reduction procedures such as midline glossectomy are used to treat people with OSA. While midline glossectomy has been described in dogs, tissue contributions to macroglossia have not been characterized. The purpose of this retrospective, descriptive, case-control study was to describe and compare volumetric dimensions of the tongue and tongue fat in brachycephalic (BC) and mesaticephalic (MC) dogs using CT images. Data collected included head and neck CT images from 17 BC and 18 control MC dogs. Multiplanar reformatted and 3D reconstructed images were created using image segmentation and specialized visualization software to calculate volumetric dimensions of the total tongue, tongue fat, and tongue muscle. Rostral and caudal topographical distributions of fat were compared. Total tongue and tongue muscle volume (P < 0.0001) and tongue fat volume (P = 0.01) normalized to body weight (BW) were greater in BC dogs. More fat was localized in the caudal tongue in both groups (P < 0.04). In regression analysis, BC conformation and increased weight were significant predictors of increased tongue fat volume. As in people, increased tongue fat may contribute to macroglossia and sleep-disordered breathing in BC dogs. Use of CT volumetry to identify tongue fat deposits may permit targeted surgical reduction of tongue volume in BC dogs and contribute substantially to treatment of BOAS.
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Obstrucción de las Vías Aéreas , Craneosinostosis , Enfermedades de los Perros , Macroglosia , Apnea Obstructiva del Sueño , Perros , Animales , Macroglosia/diagnóstico por imagen , Macroglosia/etiología , Macroglosia/cirugía , Macroglosia/veterinaria , Estudios Retrospectivos , Estudios de Casos y Controles , Lengua/diagnóstico por imagen , Apnea Obstructiva del Sueño/etiología , Apnea Obstructiva del Sueño/veterinaria , Obstrucción de las Vías Aéreas/diagnóstico por imagen , Obstrucción de las Vías Aéreas/cirugía , Obstrucción de las Vías Aéreas/veterinaria , Craneosinostosis/veterinaria , Tomografía Computarizada por Rayos X/veterinariaRESUMEN
Beckwith-Wiedemann syndrome (BWS) is a genetic disease with phenotypic variability and the following signs: macroglossia, asymmetry, lateralised overgrowth, overgrowth of the internal organs, abdominal wall defects, neonatal hypoglycemia and increased risk of embryonic tumours. The prevalence is reported as being between 1 in 10,000 and 1 in 21,000 live births. The disease is caused by molecular changes in gene clusters on the short arm of chromosome 11 region P15.5. We present the case of a female, born preterm at 32 0/7 weeks. A UPD(11)pat-mutation was diagnosed postnatally. The particular feature of her case was an early tongue reduction surgery which was necessary because of drinking and breathing difficulties. Long-lasting hypoglycemia was difficult to treat.
Asunto(s)
Síndrome de Beckwith-Wiedemann , Hipoglucemia , Macroglosia , Recién Nacido , Humanos , Femenino , Síndrome de Beckwith-Wiedemann/complicaciones , Síndrome de Beckwith-Wiedemann/diagnóstico , Síndrome de Beckwith-Wiedemann/epidemiología , Macroglosia/diagnóstico , Macroglosia/etiología , Macroglosia/cirugía , Hipoglucemia/diagnóstico , Hipoglucemia/complicacionesRESUMEN
OBJECTIVE: Macroglossia is a characteristic feature of Beckwith-Wiedemann syndrome (BWS), commonly treated with reduction glossectomy to restore form and function. There exists no consensus on the perioperative management of these patients undergoing tongue reduction surgery, including anecdotal information regarding how long postoperative intubation should be maintained. The aim of this study is to evaluate the necessity of prolonged postoperative intubation in patients receiving tongue reduction surgery via the surgical and anesthetic management methods at our center. DESIGN: Retrospective case series. SETTING: Institutional care at Level I Children's Hospital. PARTICIPANTS: All children less than 18 years old with BWS and congenital macroglossia who underwent tongue reduction surgery over 5 consecutive years at our center (N = 24). INTERVENTIONS: Tongue reduction surgery via the "W" technique. MAIN OUTCOME MEASURES: Success of immediate postoperative extubation and related surgical complications. RESULTS: Immediate, uncomplicated postoperative extubation was successfully performed in all patients who received tongue reduction surgery for congenital macroglossia. CONCLUSIONS: Prolonged postoperative intubation for tongue reduction surgery may not be necessary as immediate, uncomplicated postoperative extubation was achieved in 100% of patients who received tongue reduction surgery at our center.
Asunto(s)
Síndrome de Beckwith-Wiedemann , Macroglosia , Adolescente , Síndrome de Beckwith-Wiedemann/cirugía , Niño , Glosectomía , Humanos , Intubación Intratraqueal , Macroglosia/congénito , Macroglosia/cirugía , Estudios RetrospectivosRESUMEN
INTRODUCTION: Macroglossia occurs in 80% to 99% of patients with Beckwith-Wiedemann syndrome (BWS) and a variety of surgical techniques for tongue reduction are offered by surgeons. The purpose of this study is to evaluate the postoperative outcomes of the anterior "W" tongue reduction technique in patients with BWS. METHODS: A retrospective review was conducted of all patients diagnosed with BWS that underwent an anterior "W" tongue reduction for macroglossia in the past 7 years, performed by 2 surgeons. Demographics, procedural characteristics, perioperative outcomes, and complications were assessed. RESULTS: A total of 19 patients met inclusion criteria consisting of 8 male and 11 female patients. The mean age at the time of surgery was 405 days, mean surgeon operating time was 1.06â h, and mean length of follow-up was 467 days. Postoperative oral competence was observed in 100% of patients. There was no reported history of sleep apnea or airway compromise. Speech delay was seen in 4 patients pre- and postoperatively. Feeding issues decreased from 7 patients preoperatively to 1 patient postoperatively. Preoperative prevalence of class III malocclusion (53%) and isolated anterior open bite (26%) decreased postoperatively to 37% and 16%, respectively. The only reported complications were superficial tip wound dehiscence in 3 patients treated with nystatin antifungal therapy. None of the patients required revisional surgery. CONCLUSION: Patients treated with the anterior "W" tongue reduction technique had low rates of perioperative complications and significant improvements in oral competence. Anterior "W" tongue reduction is safe and effective for the correction of macroglossia in patients with BWS.
Asunto(s)
Síndrome de Beckwith-Wiedemann , Macroglosia , Síndrome de Beckwith-Wiedemann/complicaciones , Síndrome de Beckwith-Wiedemann/cirugía , Femenino , Glosectomía/efectos adversos , Glosectomía/métodos , Humanos , Macroglosia/congénito , Macroglosia/cirugía , Masculino , Lengua/cirugíaRESUMEN
INTRODUCTION: Macroglossia is a term used to describe a large tongue which protrudes outside of the mouth while in a resting position (Balaji, 2013). It is a cardinal sign in children with Beckwith-Wiedemann syndrome and can also be found in children with Down syndrome and Klippel-Trenaunay-Weber syndrome. Macroglossia can lead to airway and feeding difficulties, as well as problems with speech, drooling, and cosmesis. We present a review of tongue reduction operations performed for macroglossia over a 10-year period in Northern Ireland. METHODS: We performed a retrospective review of the medical notes of those children identified to have undergone a tongue reduction procedure in the regional pediatric hospital. We reviewed the presenting symptoms and concerns, the operative technique used, postoperative outcomes, and follow up. Outcomes data included improvements in symptoms, complications, and the need for revision procedures. RESULTS: Six children underwent tongue reduction procedures over a 10-year period. Age range at time of surgery was between 4 months to 10 years 3 months. Five children had an underlying diagnosis of Beckwith-Wiedemann syndrome and 1 child had Down syndrome. One child underwent a second tongue reduction for mild tongue protrusion at the 5-year follow up. There were no complications in relation to tongue reduction surgery for any of the children and importantly, there were no airway complications in our series. All patients were found to have improved feeding, better tongue position in the oral cavity, reduced drooling, and better speech development following surgery. CONCLUSION: Symptomatic macroglossia requiring a tongue reduction procedure is relatively rare and these procedures are, therefore, uncommonly performed. Despite the rarity of this procedure, when it is required, it can be life saving for some infants and children, and life altering for the remainder. Improvements in airway, feeding, speech, and psychosocial wellbeing are the desired outcomes with this procedure. Throughout our 10-year series we have found it to be a relatively safe procedure but potentially anesthetically challenging. We have demonstrated both good short and long-term outcomes for these children.
Asunto(s)
Síndrome de Beckwith-Wiedemann , Macroglosia , Síndrome de Beckwith-Wiedemann/cirugía , Niño , Glosectomía , Humanos , Lactante , Macroglosia/etiología , Macroglosia/cirugía , Estudios Retrospectivos , Lengua/cirugíaRESUMEN
Beckwith-Wiedemann syndrome (BWS) is a rare paediatric overgrowth disorder. Associated macroglossia is a feature of many children with BWS and is felt to be a risk factor for obstructive sleep apnoea (OSA). Sleep-disordered breathing is highly variable in this population. The relationship between degree of macroglossia or other genotypic or phenotypic factors and OSA severity has not been established. The natural history of OSA in this population is unknown; a variety of conservative and surgical therapies have been used to treat OSA in children with BWS but none have been studied systematically. Tongue reduction is the mainstay of surgical therapy for macroglossia associated with BWS, but limited data are available regarding its efficacy in treating OSA or its effect on speech and swallowing. More research is needed to better identify which children with BWS are at risk for OSA and the most effective treatment for these patients.
Asunto(s)
Síndrome de Beckwith-Wiedemann/complicaciones , Glosectomía/efectos adversos , Macroglosia/cirugía , Apnea Obstructiva del Sueño , Niño , Manejo de la Enfermedad , Glosectomía/métodos , Humanos , Macroglosia/etiología , Medición de Riesgo , Factores de Riesgo , Apnea Obstructiva del Sueño/epidemiología , Apnea Obstructiva del Sueño/etiología , Apnea Obstructiva del Sueño/terapiaRESUMEN
Macroglossia is classified as true macroglossia, which exhibits abnormal histology with clinical findings, and relative macroglossia, in which normal histology does not correlate with pathologic enlargement. This report describes an atypical case of morbidity with massive macroglossia secondary to myxedema; the macroglossia enlarged over a 3-month period before being presented to the Department of Oral and Maxillofacial Surgery, University of Texas Health Science Center at Houston (Houston, TX). Substantial enlargement of the tongue (16 cm long × 10 cm wide) was first attributed to angioedema, which was refractory to the discontinuation of lisinopril and a C1 esterase inhibitor. A core tongue biopsy examination was performed to rule out angioedema, amyloidosis, myxedema, and idiopathic muscular hypertrophy. Interstitial tissue was positive for Alcian blue and weakly positive for colloidal iron, which are correlated with hypothyroidism and a diagnosis of myxedema. However, the macroglossia did not resolve after correcting for hypothyroidism. The patient required a wedge glossectomy for definitive treatment. She recovered unremarkably, with excellent cosmesis and preservation of lingual and hypoglossal function. There are some case reports of massive macroglossia but none with myxedema as the primary etiology.
Asunto(s)
Glosectomía/métodos , Hipotiroidismo/complicaciones , Macroglosia/etiología , Macroglosia/cirugía , Mixedema/complicaciones , Adulto , Femenino , Humanos , Accidente Cerebrovascular/cirugíaAsunto(s)
Macroglosia , Glosectomía , Humanos , Macroglosia/congénito , Macroglosia/etiología , Macroglosia/patología , Macroglosia/cirugía , LenguaRESUMEN
The macroglossia is a rare condition, congenital or acquired, characterized by hypertrophy of the lingual muscles that can cause both aesthetic and functional changes such as mandibular prognathism and malocclusion. Diagnosis is through clinical examination. Treatment consists of excision of part of the tongue and different surgical techniques have been described in the literature. The keyhole lingual resection technique has shown satisfactory results in reducing the volume and preservation of the neurovascular bundles of the tongue. This work aims to present a clinical report of true macroglossia associated with dental-skeletal discrepancies, submitted to partial glossectomy previously to orthognathic surgery.
Asunto(s)
Glosectomía/métodos , Macroglosia/cirugía , Maloclusión de Angle Clase III/cirugía , Estética Dental , Humanos , Masculino , Lengua/cirugía , Adulto JovenRESUMEN
The tongue is a vital human organ that needs to be checked before beginning a dental treatment, especially one in orthodontics or sleep apnea. The size of the tongue is an issue because there is not yet a clear definition of what a normal-sized tongue is. This leads to further problems in defining macroglossia. The current article aims to define macroglossia and bringforth more information about the two different types: pseudo macroglossia and true macroglossia. It further discusses treatment solutions available, such as partial glossectomies, coblation technique, and radiofrequency waves treatment.
Asunto(s)
Glosectomía/métodos , Macroglosia/cirugía , Humanos , Macroglosia/etiología , Lengua/anatomía & histología , Lengua/fisiologíaRESUMEN
Macroglossia is a rare condition, but can severely affect the oral and maxillofacial region. Angioedema is a condition resulting from multiple mechanisms, all of which can result in macroglossia. This report describes an unusual case of acquired macroglossia in an adult resulting from chronic edema secondary to angioedema in the setting of stroke. The patient had a morbidly enlarged tongue and presented with clinical signs and symptoms consistent with massive macroglossia. She required surgical intervention for acute management of her symptoms and definitive treatment of her macroglossia.
Asunto(s)
Angioedema/complicaciones , Macroglosia/etiología , Femenino , Humanos , Macroglosia/cirugía , Persona de Mediana Edad , Accidente Cerebrovascular/complicaciones , Accidente Cerebrovascular/radioterapia , Tomografía Computarizada por Rayos XRESUMEN
BACKGROUND: Macroglossia has been reported in patients undergoing posterior fossa neurosurgical procedures and is thought to be as a result of venous engorgement from intubation or mechanical positioning during these prolonged procedures. METHODS: We report three patients who developed macroglossia and dysautonomia of central neurogenic origin following brainstem injury. RESULTS: The three patients developed macroglossia and dysautonomia with wide hemodynamic fluctuations in the setting of posterior fossa injury of the lower brainstem structures, necessitating tracheostomy placement. Macroglossia was managed with dexamethasone and there was complete resolution of dysautonomia while treated with beta-blockers and gabapentin. CONCLUSIONS: Neurointensivists should be aware of macroglossia with dysautonomia complicating brainstem injury, which may have perilous consequences in the setting of cerebral edema or intracranial hypertension.
Asunto(s)
Lesiones Encefálicas/complicaciones , Tronco Encefálico/lesiones , Macroglosia/etiología , Adolescente , Antagonistas Adrenérgicos beta/uso terapéutico , Anciano , Aminas/uso terapéutico , Antiinflamatorios/uso terapéutico , Lesiones Encefálicas/etiología , Bloqueadores de los Canales de Calcio/uso terapéutico , Fosa Craneal Posterior/lesiones , Ácidos Ciclohexanocarboxílicos/uso terapéutico , Dexametasona/uso terapéutico , Femenino , Gabapentina , Humanos , Macroglosia/tratamiento farmacológico , Macroglosia/cirugía , Masculino , Persona de Mediana Edad , Disautonomías Primarias/tratamiento farmacológico , Disautonomías Primarias/etiología , Traqueostomía/métodos , Resultado del Tratamiento , Ácido gamma-Aminobutírico/uso terapéuticoRESUMEN
Beckwith-Wiedemann syndrome (BWS) is a congenital growth disorder. Children born with BWS develop enlarged organs, including the tongue, a large body, and other signs. A woman with BWS was treated and followed for 30 years. Treatment consisted of tongue reduction, orthopedic and orthodontic treatment, orthognathic surgery, and retention. The patient was first treated when she was 5 years old. Her original orthodontic problems included macroglossia, anterior open bite, anterior crossbite, and a skeletal Class III jaw relationship caused by significant mandibular protrusion. The jaw-base relationships did not improve in the early preadolescent period after phase 1 of orthodontic treatment with a vertical chincap. With the growth spurt accompanying puberty, she developed a severe skeletal Class III jaw relationship and a constricted maxillary arch. Surgically assisted rapid maxillary expansion was performed at 23 years of age to correct the severe discrepancy between the maxillary and mandibular dental arch widths. Then, at 26 years, a LeFort I osteotomy, a horseshoe osteotomy, a bilateral sagittal split ramus osteotomy, and genioplasty were performed after presurgical orthodontic treatment with extraction of the mandibular first molars. Both the facial profile and the occlusion were stable after 6 years of retention. This case report discusses the result of long-term observation of a patient with BWS who underwent tongue reduction, early orthodontic treatment, and surgical-orthodontic treatment.
Asunto(s)
Síndrome de Beckwith-Wiedemann/terapia , Ortodoncia Correctiva/métodos , Procedimientos Quirúrgicos Ortognáticos/métodos , Síndrome de Beckwith-Wiedemann/cirugía , Preescolar , Aparatos de Tracción Extraoral , Femenino , Estudios de Seguimiento , Mentoplastia/métodos , Glosectomía/métodos , Humanos , Estudios Longitudinales , Macroglosia/cirugía , Maloclusión de Angle Clase III/cirugía , Maloclusión de Angle Clase III/terapia , Maxilar/anomalías , Mordida Abierta/cirugía , Mordida Abierta/terapia , Osteotomía Le Fort/métodos , Osteotomía Sagital de Rama Mandibular/métodos , Técnica de Expansión Palatina , Planificación de Atención al Paciente , Prognatismo/cirugía , Prognatismo/terapia , Resultado del TratamientoRESUMEN
Lymphangiomas are rare congenital malformations which are commonly seen in the head and neck region. The disease can be histologically differentiated from other vascular disorders such as cavernous or capillary hemangioma with the lymphatic endothelium-lined cystic spaces. The onset of lymphangiomas are either at birth (60 to 70%) or up to two years of age (90%). It is rare in adults. The therapeutic strategy is mainly based on the surgical removal of the lesion. The risk of recurrence is high in incomplete removal of the lesion. In this article, we discuss the major clinical manifestations, disease-related impairments and dental problems which patients may face as well as treatment options for lymphangioma of the tongue. Within this context, we present four cases of lymphangioma of tongue, including two with a giant macroglossia leading problems in dentition and related dental problems.
Asunto(s)
Linfangioma/diagnóstico , Macroglosia/congénito , Recurrencia Local de Neoplasia/diagnóstico , Neoplasias de la Lengua/diagnóstico , Adulto , Niño , Diagnóstico Diferencial , Femenino , Humanos , Linfangioma/patología , Linfangioma/cirugía , Macroglosia/diagnóstico , Macroglosia/patología , Macroglosia/cirugía , Masculino , Recurrencia Local de Neoplasia/patología , Recurrencia Local de Neoplasia/cirugía , Neoplasias de la Lengua/patología , Neoplasias de la Lengua/cirugíaRESUMEN
The aim of this study was to evaluate the postoperative course and long-term functional and aesthetic outcomes in patients with Beckwith-Wiedemann syndrome (BWS) following surgical reduction of macroglossia, using multiple questionnaires. Patients with BWS who underwent keyhole reduction for macroglossia were included in this study. The postoperative course for each patient was recorded, and multiple questionnaires were administered to evaluate aesthetic concerns, oral incompetence or feeding difficulties, sleep-disordered breathing symptoms, and speech. Nine patients underwent ten reduction glossoplasty surgeries. The mean age at surgery was 22 months. The postoperative course for each case was uneventful, except for one patient who had wound dehiscence. The questionnaires revealed significant improvements in tongue appearance, feeding, drooling, facial appearance, and psychosocial outcomes. There was also a significant reduction in sleep-disordered breathing symptoms after surgery. Keyhole reduction glossoplasty is a safe and effective procedure for the treatment of macroglossia in BWS patients, with excellent functional and aesthetic outcomes and a low complication rate.